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Myeloproliferative disorders

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https://www.readbyqxmd.com/read/29790256/platelet-characteristics-in-patients-with-essential-thrombocytosis
#1
Oliver Heidmann Pedersen, Mads Lamm Larsen, Erik Lerkevang Grove, Peter Buur van Kooten Niekerk, Søren Bønløkke, Peter H Nissen, Steen Dalby Kristensen, Anne-Mette Hvas
BACKGROUND: Essential thrombocytosis (ET) is a myeloproliferative disorder characterized by an increased platelet count. ET is associated with an increased risk of thrombosis, and procoagulant features of the disease may include an increased number of reactive reticulated platelets and an increased aggregation potential. We aimed to explore the association between platelet count, platelet turnover and platelet aggregation in patients with ET. METHODS: We included 24 ET patients who discontinued antiplatelet therapy prior to blood sampling...
May 23, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29774106/hematopoietic-restricted-ptpn11e76k-reveals-indolent-mpn-progression-in-mice
#2
Stefan P Tarnawsky, Wen-Mei Yu, Cheng-Kui Qu, Rebecca J Chan, Mervin C Yoder
Juvenile Myelomonocytic Leukemia (JMML) is a pediatric myeloproliferative neoplasm (MPN) that has a poor prognosis. Somatic mutations in Ptpn11 are the most frequent cause of JMML and they commonly occur in utero . Animal models of mutant Ptpn11 have probed the signaling pathways that contribute to JMML. However, existing models may inappropriately exacerbate MPN features by relying on non-hematopoietic-restricted Cre-loxP strains or transplantations into irradiated recipients. In this study we generate hematopoietic-restricted models of Ptpn11E76K-mediated disease using Csf1r-MCM and Flt3Cre...
April 24, 2018: Oncotarget
https://www.readbyqxmd.com/read/29773831/dnajc17-is-localized-in-nuclear-speckles-and-interacts-with-splicing-machinery-components
#3
A Pascarella, G Ferrandino, S C Credendino, C Moccia, F D'Angelo, B Miranda, C D'Ambrosio, P Bielli, O Spadaro, M Ceccarelli, A Scaloni, C Sette, M De Felice, G De Vita, E Amendola
DNAJC17 is a heat shock protein (HSP40) family member, identified in mouse as susceptibility gene for congenital hypothyroidism. DNAJC17 knockout mouse embryos die prior to implantation. In humans, germline homozygous mutations in DNAJC17 have been found in syndromic retinal dystrophy patients, while heterozygous mutations represent candidate pathogenic events for myeloproliferative disorders. Despite widespread expression and involvement in human diseases, DNAJC17 function is still poorly understood. Herein, we have investigated its function through high-throughput transcriptomic and proteomic approaches...
May 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29749755/t784c-lnk-gene-polymorphism-and-the-risk-of-myeloproliferative-disorders
#4
Igor A Olkhovskiy, Aleksey S Gorbenko, Marina A Stolyar, Evgeniy V Vasiliev, Mikhail A Mikhalev, Kseniya A Tabakova
No abstract text is available yet for this article.
May 11, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29745886/primary-myelofibrosis-but-not-autoimmune-myelofibrosis-accompanied-by-sj%C3%A3-gren-s-syndrome-and-primary-biliary-cirrhosis-in-a-patient-with-trisomy-8-mosaic-a-case-report-and-literature-review
#5
Chenyang Lu, Xiaoyan Wu, Hongyan Wen, Huiying Gao, Caihong Wang, Bo Yang, Zhipeng Liang, Chong Gao, Xiaofeng Li
Bone marrow fibrosis has been found to be associated with autoimmune disorders, and autoimmune myelofibrosis (AIMF) has been defined. Primary myelofibrosis (PMF), a clonal myeloproliferative disorder, should be distinguished from AIMF which has a good response to steroids, as the former has a high mortality and very bad response to conventional treatment. This case report describes a rare case of PMF accompanied with Sjögren's syndrome (SJS) and primary biliary cirrhosis (PBC), in a patient with trisomy 8 mosaic...
May 10, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29741513/myelofibrosis-clinicopathologic-features-prognosis-and-management
#6
Jennifer M O'Sullivan, Claire N Harrison
Myelofibrosis is one of the BCR-ABL-negative clonal disorders that collectively are known as myeloproliferative neoplasms (MPNs). It is caused by the proliferation of clonal hematopoietic stem cells, which over time leads to characteristic clinical features. The disease presentation is heterogeneous, however, with 30% of patients initially asymptomatic. This variation in clinical phenotype warrants careful risk stratification to guide appropriate management, and prognostic risk scores are continually being refined...
February 2018: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29730367/peripheral-artery-disease-associated-with-myeloproliferative-disorders
#7
Shoko Nakagawa, Osami Kawarada, Takeshi Yagyu, Jiro Matsuo, Yosuke Inoue, Teruo Noguchi, Satoshi Yasuda
No abstract text is available yet for this article.
April 27, 2018: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/29724146/chronic-myeloproliferative-disorders-is-quality-of-life-the-new-goal
#8
Elisabetta Abruzzese, Pasquale Niscola, Malgorzata Monika Trawinska, Paolo de Fabritiis
No abstract text is available yet for this article.
May 3, 2018: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/29705369/essential-thrombocythemia-and-cardiac-surgery-a-case-series-and-review-of-the-literature
#9
Carmelina Gurrieri, Bradford B Smith, Gregory A Nuttall, Rajiv K Pruthi, Sameh M Said, Mark M Smith
BACKGROUND: Essential thrombocythemia (ET) is a rare myeloproliferative disorder characterized by an unexplained thrombocytosis (>450 x109 /L) and associated vasomotor, thrombotic and hemorrhagic manifestations. While the literature detailing the perioperative management of patients with ET undergoing cardiac surgery is sparse, major perioperative complications have been reported, particularly in poorly controlled patients presenting with platelet counts ≥800 x 109 /L. The purpose of this study was to provide the experience at a large tertiary medical center in managing patients with ET undergoing cardiac surgery, and to summarize the available literature...
April 26, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29686565/chronic-neutrophilic-leukemia
#10
Arthur Bredeweg, Micah Burch, John R Krause
Chronic neutrophilic leukemia is a rare myeloproliferative disorder characterized by a sustained peripheral blood neutrophilia, absence of the BCR/ABL oncoprotein, bone marrow hypercellularity with less than 5% myeloblasts and normal neutrophil maturation, and no dysplasia. This leukemia has been associated with mutations in the colony-stimulating factor 3 receptor (CSF3R) that may activate this receptor, leading to the proliferation of neutrophils that are the hallmark of chronic neutrophilic leukemia. We present a case of chronic neutrophilic leukemia and discuss the criteria for diagnosis and the significance of mutations found in this leukemia...
January 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29684340/myeloid-sarcoma-with-megakaryoblastic-differentiation-presenting-as-a-breast-mass
#11
Ifeyinwa E Obiorah, Metin Ozdemirli
Myeloid sarcoma is an extramedullary tumor that consists of myeloblasts or immature myeloid cells. The neoplasm can occur in any part of the body, including the bone, periosteum, lymph nodes, skin, and soft tissue and they may occur de novo or in association with acute myeloid leukemia, myeloproliferative neoplasms and myelodysplastic syndromes. Most cases display a myelomonocytic or pure monoblastic morphology. Tumors with megakaryoblastic differentiation are extremely uncommon and may occur in association with transformation of a myeloproliferative disorder...
April 17, 2018: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/29683036/assessment-of-endothelial-dysfunction-with-flow-mediated-dilatation-in-myeloproliferative-disorders
#12
Abdulkerim Yildiz, Melike Güryildirim, Mehmet Sezgin Pepeler, Merve Yazol, Suna Özhan Oktar, Kadir Acar
BACKGROUND: Thrombosis is the most important cardiovascular complication of classical myeloproliferative disorders (MPDs). Endothelial dysfunction (ED) is known to play a major role in the mechanism of thrombophilia in MPDs. METHODS: Endothelial dysfunction and its associations with other parameters were investigated. A total of 18 patients with polycythemia vera (PV), 24 with essential thrombocytosis (ET), 7 with primary myelofibrosis (PMF), and 30 healthy patients as a control group were included in the study...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29676020/chronic-neutrophilic-leukemia-an-extremely-rare-cause-of-neutrophilia-in-childhood-cure-with-hematopoietic-stem-cell-transplantation
#13
Vedat Uygun, Hayriye Daloğlu, Seda Öztürkmen, Gülsün Karasu, Zekai Avcı, Akif Yeşilipek
CNL is a rare myeloproliferative disorder frequently seen in older adults. A significant proportion of patients show progression to AML. Here, we report the case of a patient with FA who was monitored for leukopenia but who developed leukocytosis during the follow-up and was diagnosed with CNL probably after an acquired CSF3R mutation. Because the patient had FA, which could accelerate the progression to AML, an HSCT was performed, which resulted in cure. This patient (aged 12 years) is one of the youngest patients reported to develop CNL as well as the first FA patient with a diagnosis of CNL...
April 19, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29670795/pulmonary-vasculitis-and-a-horseshoe-kidney-in-noonan-syndrome
#14
Surasak Puvabanditsin, Rosanna Abellar, Adaora Madubuko, Rajeev Mehta, Lauren Walzer
We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp) pathogenic variant in PTPN11 . The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy. This report presents a new case of Noonan syndrome with unusual associated disorders and a review of the literature...
2018: Case Reports in Pathology
https://www.readbyqxmd.com/read/29665937/-current-understanding-of-myeloproliferative-neoplasm-related-gene-mutations-and-cytokine-review
#15
Zhi-Peng He, Yong Wu
Myeloproliferative neoplasm(MPN) is clonal hematopoietic stem cell disorder characterized by abnormal proliferation and expansion of one or more myeloid lineages. BCR-ABL-negative MPN includes polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The mutations of JAK2, CALR and MPL genes are involved in the pathogenesis of MPN that provided a more complete molecular diagnostic standard for MPN. More and more new mutated genes related to prognosis of MPN were discovered in the past few years, at same time it was found that cytokines were also involved in the genesis and development of MPN...
April 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29665657/recent-advances-in-the-diagnosis-and-management-of-primary-myelofibrosis
#16
Katsuto Takenaka, Kazuya Shimoda, Koichi Akashi
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) in which dysregulation of the Janus kinase/signal transducers and activators of transcription (JAK/STAT) signaling pathways is the major pathogenic mechanism. Most patients with PMF carry a driver mutation in the JAK2, MPL (myeloproliferative leukemia), or CALR (calreticulin) genes. Mutations in epigenetic regulators and RNA splicing genes may also occur, and play critical roles in PMF disease progression. Based on revised World Health Organization diagnostic criteria for MPNs, both screening for driver mutations and bone marrow biopsy are required for a specific diagnosis...
April 20, 2018: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29662245/early-human-cytomegalovirus-reactivation-is-associated-with-lower-incidence-of-relapse-of-myeloproliferative-disorders-after-allogeneic-hematopoietic-stem-cell-transplantation
#17
Zinaida Peric, Jonas Wilson, Nadira Durakovic, Alen Ostojic, Lana Desnica, Violeta Rezo Vranjes, Ivana Marekovic, Ranka Serventi-Seiwerth, Radovan Vrhovac
Conflicting results have been reported regarding the association between early cytomegalovirus (CMV) reactivation and relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT). This prompted us to evaluate the impact of CMV reactivation on outcomes of 155 consecutive adult patients transplanted in our institution. In our study, CMV reactivation did not affect cumulative incidence (CI) of relapse in patients with lymphoproliferative disorders. However, the CI of relapse in patients with myeloproliferative disorders (AML and MPN) was 37% (95% CI, 21-53) in patients without CMV reactivation as opposed to 17% (95% CI, 9-28) in patients with CMV reactivation (p = 0...
April 16, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29658352/chronic-subdural-collection-overlying-an-intra-axial-hemorrhagic-lesion-in-chronic-myelomonocytic-leukemia-special-report-and-review-of-the-literature
#18
Anne-Laure Bernat, Stefano Maria Priola, Ahmad Elsawy, Faisal Farrash, Shervin Taslimi, Fred Gentili
Chronic myelomonocytic leukaemia (CMML) is a clonal hematopoietic stem cell disorder characterized by the presence of an absolute monocytosis in the peripheral blood (>1 x 109 /L) and the presence of myelodysplastic and myeloproliferative features in the bone marrow. Involvement of the central nervous system (CNS) is uncommon in CMML. Areas covered: Herein described is a case report of a CMML patient who presents with symptomatic chronic subdural collection overlying a haemorrhagic brain lesion, along with diffuse dural infiltration, after two cycles of azacytidine...
April 19, 2018: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29643232/increased-neutrophil-extracellular-trap-formation-promotes-thrombosis-in-myeloproliferative-neoplasms
#19
Ofir Wolach, Rob S Sellar, Kimberly Martinod, Deya Cherpokova, Marie McConkey, Ryan J Chappell, Alexander J Silver, Dylan Adams, Cecilia A Castellano, Rebekka K Schneider, Robert F Padera, Daniel J DeAngelo, Martha Wadleigh, David P Steensma, Ilene Galinsky, Richard M Stone, Giulio Genovese, Steven A McCarroll, Bozenna Iliadou, Christina Hultman, Donna Neuberg, Ann Mullally, Denisa D Wagner, Benjamin L Ebert
Thrombosis is a major cause of morbidity and mortality in Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), clonal disorders of hematopoiesis characterized by activated Janus kinase (JAK)-signal transducer and activator of transcription (STAT) signaling. Neutrophil extracellular trap (NET) formation, a component of innate immunity, has been linked to thrombosis. We demonstrate that neutrophils from patients with MPNs are primed for NET formation, an effect blunted by pharmacological inhibition of JAK signaling...
April 11, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29622863/summary-and-review-of-the-abstracts-on-philadelphia-negative-myeloproliferative-neoplasms-presented-at-haematocon-2017
#20
Tathagata Chatterjee, Ankur Ahuja
There are lot of grey zones in Philadelphia negative chronic myeloproliferative neoplasms (CMPNs) and that's the reason they are in hit list of researchers. Having a spectrum of disorders their diagnosis is very important and especially to differentiate from each other since they overlap with each other in many ways. Diagnosis doesn't start from lab but with clinical phenotype. Clinical phenotype not only able to provide us the diagnosis but also helps in management of the disease per se. When diagnosis comes, the old timer but an evergreen morphology plays an important role which along with the newer generation tool "molecular" helps in differentiating these disorders...
April 2018: Indian Journal of Hematology & Blood Transfusion
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